Drug-Induced Pseudoporphyria: A Case Report.

Pseudoporphyria is an uncommon dermatosis resembling porphyria cutanea tarda (PCT). The exclusion of true porphyria, especially PCT, is critically essential for diagnosing pseudoporphyria. It has an unknown underlying pathophysiology with a normal or near-normal porphyrin profile. Pseudoporphyria has been associated with chronic renal failure and hemodialysis, medications, and tanning beds. In drug-induced pseudoporphyria cases, eliminating the suspected photosensitizing drug improves the disease typically within weeks to months (on average eight weeks). In genetically predisposed individuals, phototoxic metabolites may trigger the development of skin fragility, bullae, milia, and scarring on the dorsum of the hands and other sun-exposed areas. Wearing a broad-spectrum sunscreen and maintaining strict ultraviolet protection is essential in cases of pseudoporphyria. We report the case of a 20-year-old male who presented to us with complaints of photosensitivity and multiple erosions with irregular scars over photo-exposed areas involving the dorsum of the hands and face predominantly. The patient was evaluated further to determine the underlying cause. A wood's lamp examination of the urine was done, which did not show fluorescence. Based on clinical and laboratory findings, the diagnosis of pseudoporphyria was made, and the patient was started on the oral antimalarial agent hydroxychloroquine sulfate with strict sun protection.

Dermatological Manifestations in Patients With Chronic Kidney Disease: A Review.

Chronic kidney disease (CKD) is a progressive disease and has multiple clinical manifestations; when CKD reaches the end stage, at least one cutaneous manifestation appears due to some increased toxin levels or a constant proinflammatory state. Nonspecific manifestations include pruritus, xerosis, pigmentation disorders, acquired ichthyosis, purpuric spots, and nail disorders. Some specific manifestations are bullous dermatoses, acquired perforating dermatoses (APD), eruptive xanthoma, access site infections, calcifying disorders, and nephrogenic systemic fibrosis (NSF). All these cutaneous changes negatively impact patients; early recognition and diagnosis of these dermatoses will make a difference in their quality of treatment. Exploring a patient's skin is fundamental to suspect some diseases and increased toxin levels; pruritus occurs when uremic toxins are raised, and nail disorders are associated with hypoalbuminemia. This review provides the clinician with information on the clinical manifestations that occur in CKD, including epidemiology, pathophysiology, clinical manifestations, diagnosis, histopathology, treatment, and life impact of the dermatoses in CKD.

Renal Transplantation Could Reverse Dialysis-Associated Porphyria.

Pseudoporphyria (PP) is a relatively infrequent, photodistributed bullous dermatosis that clinically, histopathologically, and immunologically resembles porphyria cutanea tarda (PCT), but is not accompanied by porphyrin abnormalities in the serum, urine, or stool. It was initially described in patients with renal failure on dialysis as 'bullous dermatosis of hemodialysis.' Pseudoporphyria has been seen in patients with end-stage renal disease on hemodialysis. No treatment has proved efficacious in the management of pseudoporphyria. However, N-acetylcysteine has been anecdotally reported to be effective in the management of hemodialysis-related pseudoporphyria and other porphyric diseases. Our patient had developed multiple skin lesions all over the body when hemodialysis started. The lesions were erythematous with fluid-filled vesicles, and bullae with cutaneous fragility that were evaluated and diagnosed as pseudoporphyria. The patient was treated with available all medication in the literature but was not relieved. However, all skin lesions completely healed within 22 days post renal transplantation. Renal transplantation proved to be the cure for dialysis-induced pseudoporphyria resistant to conventional drug therapy.

Direct Immunofluorescence of Mechanobullous Epidermolysis Bullosa Acquisita, Porphyria Cutanea Tarda and Pseudoporphyria.

Mechanobullous epidermolysis bullosa acquisita (mEBA) can have a clinical presentation that is very similar to other blistering diseases, such as porphyria cutanea tarda (PCT) and pseudoporphyria. Direct immunofluorescence is an important feature in the diagnosis of mEBA, although features that overlap with PCT and pseudoporphyria have been reported. This retrospective observational study investigated whether direct immunofluorescence can discriminate mEBA from PCT and pseudoporphyria. Biopsies of 13 patients with mEBA, 10 with PCT and 10 with pseudoporphyria were included. In 7 cases of PCT and 4 of pseudoporphyria, direct immunofluorescence showed a pattern at the dermal-epidermal junction that appeared similar to the u-serrated pattern in mEBA. Vessel wall depositions were observed in all 3 diseases, but were more frequent and more intense in PCT and pseudoporphyria than in mEBA. Careful examination of direct immunofluorescence of mEBA vs. PCT and pseudoporphyria revealed different staining patterns, although overlapping features were present. Therefore, integrating all clinical and laboratory data is essential to differentiate between mEBA, PCT and pseudoporphyria.

Dialysis-associated Pseudoporphyria: An Often Overlooked Cause

Pseudoporphyria is a condition clinically and histologically similar to porphyria cutanea tarda (PCT)but without abnormalities in porphyrin metabolism. Pseudoporphyria has also been described inpatients with chronic renal failure, with or without accompanying dialysis. Herein we report a caseof dialysis-associated pseudoporphyria in the hopes that increased awareness of this condition mayultimately lead to improved outcomes with the institution of specific treatment measures.

Acquired reactive perforating collagenosis and pseudoporphyric bullous dermatosis in a hemodialysis patient.

Hemodialysis patients present with a broad spectrum of specific and nonspecific skin disorders, which rarely coexist. We report an exceptional case of a hemodialysis patient that developed acquired reactive perforating collagenosis and pseudoporphyric bullous dermatosis on the basis of common skin disorders which include hyperpigmentation, pruritus, xerosis cutis, and Linsday's nails. Interestingly, our patient presented with two unusual but distinctive cutaneous dermopathies on the background of other commonly seen skin alterations. The patient was successfully treated with allopurinol and N-acetylcysteine. Avoidance of potentially triggering factors such as alcohol, sunlight exposure and certain medication was recommended. Thus, increasing clinical awareness, assiduous investigation and early treatment of skin disorders are required to improve the prognosis and quality of life in this patient population.

Drug induced pseudoporphyria in CKD: A case report.

Pseudoporphyria (PP) is used to describe a photodistributed bullous disorder with clinical and histologic features of porphyria cutanea tarda (PCT) but without accompanying biochemical porphyrin abnormalities. Medications, excessive sun and ultraviolet radiation exposure, have all been reported to develop PP. We report a case of PP in a 49-year-old man with CKD stage 3a, caused due to torsemide intake. This is probably the first reported case of PP developing in a dialysis naive patient CKD due to torsemide intake from India.

Phototoxicity, Pseudoporphyria, and Photo-onycholysis Due to Voriconazole in a Pediatric Patient With Leukemia and Invasive Aspergillosis.

Voriconazole is a triazole antifungal agent superior to amphotericin B in the treatment of invasive aspergillosis. It is generally well tolerated and has excellent oral bioavailability, providing significant benefit in the treatment of invasive fungal infections. There have been numerous reports of dermatologic reactions to this agent, including erythroderma, cheilitis, Stevens-Johnson syndrome, discoid lupus erythematosus, pseudoporphyria, squamous cell carcinoma, and photosensitivity reactions. Pseudoporphyria, a dermatologic condition mimicking porphyria cutanea tarda, has been described as an adverse effect of voriconazole use. Clinical findings include photosensitivity, vesicles, bullae, milia, and scarring in sun-exposed areas. Photo-onycholysis is a phenomenon of nail discoloration and onycholysis that has been described in the setting of a phototoxic drug reaction and pseudoporphyria. Implicated drugs have most commonly been tetracyclines, fluoroquinolones, and psoralens; others have been reported as well. We report a case of a pediatric patient with leukemia who developed symptoms consistent with pseudoporphyria and later photo-onycholysis while being treated with voriconazole. To our knowledge, this is the first reported case of pseudoporphyria due to voriconazole in a pediatric patient and the first reported case of photo-onycholysis as a consequence of voriconazole use.

Pseudoporphyria following self-medication with chlorophyll.

Two cases of pseudoporphyria are described in which the clinical features of porphyria cutanea tarda occurred in the absence of abnormalities in porphyrin metabolism. Both patients presented with skin fragility and bullae on the dorsal aspect of the hands. The patients consumed a commercial liquid chlorophyll drink in which we detected fluorescent compounds with characteristics typical of previously described chlorophyll derived photosensitisers.

A Case of Pseudoporphyria / 대한피부과학회지

Pseudoporphyria is a generic term that is used to describe photoaggravated bullous dermatoses associated with multiple iatrogenic causes, including medications and dialysis. The bullous lesions of pseudoporphyria are similar to those of porphyria cutanea tarda, both clinically and histologically, but they occur in the absence of the abnormally high levels of porphyrins which are found in true porphyrias. Treatment entails discontinuation of suspected agents and sun protection, especially against UVA wavelengths. We report a case of pseudoporphyria in a 28-year-old male who had erythematous crusted erosions, vesicles and hyperpigmented macules on his face and both forearms. A biopsy from his Lt. forearm revealed subepidermal bullaes with festooning of dermal papillae, and mild lymphocytic perivascular infiltrations. However laboratory tests for porphyrin were negative in his urine, blood and stool.